Update on Takayasu arteritis: Year in review 2024.

Takayasu arteritis is an uncommon systemic inflammatory large vessel vasculitis affecting women in their third and fourth decades frequently. The disease poses considerable morbidity and mortality owing to the involvement of the aorta and its major branches. Treatment comprises medical and vascular interventions, tailored to each patient. We review the high-impact publications of the year 2023 up to April 2024, which provide great insight into clinical, biomarker, imaging, pathogenetic, and therapeutic updates.

A Glimpse into Humoral Response and Related Therapeutic Approaches of Takayasu's Arteritis.

Takayasu's arteritis (TAK) manifests as an insidiously progressive and debilitating form of granulomatous inflammation including the aorta and its major branches. The precise etiology of TAK remains elusive, with current understanding suggesting an autoimmune origin primarily driven by T cells. Notably, a growing body of evidence bears testimony to the widespread effects of B cells on disease pathogenesis and progression. Distinct alterations in peripheral B cell subsets have been described in individuals with TAK. Advancements in technology have facilitated the identification of novel autoantibodies in TAK. Moreover, emerging data suggest that dysregulated signaling cascades downstream of B cell receptor families, including interactions with innate pattern recognition receptors such as toll-like receptors, as well as co-stimulatory molecules like CD40, CD80 and CD86, may result in the selection and proliferation of autoreactive B cell clones in TAK. Additionally, ectopic lymphoid neogenesis within the aortic wall of TAK patients exhibits functional characteristics. In recent decades, therapeutic interventions targeting B cells, notably utilizing the anti-CD20 monoclonal antibody rituximab, have demonstrated efficacy in TAK. Despite the importance of the humoral immune response, a systematic understanding of how autoreactive B cells contribute to the pathogenic process is still lacking. This review provides a comprehensive overview of the biological significance of B cell-mediated autoimmunity in TAK pathogenesis, as well as insights into therapeutic strategies targeting the humoral response. Furthermore, it examines the roles of T-helper and T follicular helper cells in humoral immunity and their potential contributions to disease mechanisms. We believe that further identification of the pathogenic role of autoimmune B cells and the underlying regulation system will lead to deeper personalized management of TAK patients. We believe that further elucidation of the pathogenic role of autoimmune B cells and the underlying regulatory mechanisms holds promise for the development of personalized approaches to managing TAK patients.

Current evidence on the role of fibroblasts in large-vessel vasculitides: From pathogenesis to therapeutics.

Large-vessel vasculitides (LVV) comprise a group of chronic inflammatory diseases of the aorta and its major branches. The most common forms of LVV are giant cell arteritis (GCA) and Takayasu arteritis (TAK). Both GCA and TAK are characterized by granulomatous inflammation of the vessel wall accompanied by a maladaptive immune and vascular response that promotes vascular damage and remodeling. The inflammatory process in LVV starts in the adventitia where fibroblasts constitute the dominant cell population. Fibroblasts are traditionally recognized for synthesizing and renewing the extracellular matrix thereby being major players in maintenance of normal tissue architecture and in tissue repair. More recently, fibroblasts have emerged as a highly plastic cell population exerting various functions, including the regulation of local immune processes and organization of immune cells at the site of inflammation through production of cytokines, chemokines and growth factors as well as cell-cell interaction. In this review, we summarize and discuss the current knowledge on fibroblasts in LVV. Furthermore, we identify key questions that need to be addressed to fully understand the role of fibroblasts in the pathogenesis of LVV.

[Chinese guideline for the diagnosis and treatment of Takayasu's arteritis(2023)].

Takayasu's arteritis (TAK) is a chronic granulomatous inflammatory disease that involves the aorta and its primary branch arteries. It is characterized by wall thickening,stenosis or aneurysm formation of involved arteries,leading to ischemia of related organs. The clinical diagnosis and treatment of TAK is challenging. In order to further improve the level of diagnosis and treatment of TAK in China and standardize the diagnosis and treatment of TAK, a clinical practice guidelines based on evidence-based medicine evidence was developed under the leadership of the National Clinical Medical Research Center for Dermatologic and Immunologic Diseases. Eleven recommendations for 11 clinical questions problems that are important to the diagnosis and treatment of TAK were developed based on the latest research evidence and expert opinions, combined with real clinical practice in China.

Bibliometric analysis and visualisation of research hotspots and frontiers in Takayasu's arteritis.

OBJECTIVES: Takayasu's arteritis (TAK) is an uncommon granulomatous large-vessel vasculitis associated with significant morbidity and mortality. This study endeavours to comprehend the research status and future frontiers through a bibliometric analysis. METHODS: Relevant original articles published in English were acquired from the Science Citation Index Expanded of the Web of Science Core Collection. Analysis of countries/regions, institutions, authors, co-cited references, and keywords were done using Citespace and VOSviewer software. RESULTS: The final analysis included 2215 documents contributed by 9091 scholars from 2053 institutions in 83 countries, with the United States being the largest contributor globally. Institutional and author collaboration analysis showed that collaborations are scattered and lack stable and intensive collaborative relationships. The journal 'Clinical and Experimental Rheumatology' was the most prolific journal. Anti-endothelial cell antibodies, interleukin-6, adalimumab, colour Doppler ultrasonography, and stents and prosthesis were the main research areas in TAK.Double-blind multicentre clinical trials, disease activity evaluation and cytokines were identified to be recent keyword bursts. CONCLUSIONS: Although the area of TAK research is growing rapidly, intensive institutional and author collaboration has to be fostered in the future to fuel TAK research and information dissemination. Future research on TAK may revolve around cytokines, disease activity evaluation and clinical trials.

Does Bronchial Artery Hypertrophies After Descending Thoracic Aorta Stenting in Takayasu Arteritis? - A Clinical Dilemma.

41-year-old lady, known case of Takayasu arteritis with pulmonary arterial involvement, presented with multiple episodes of haemoptysis (maximum 50 mL) in a week. She had undergone descending thoracic aorta angioplasty and stenting 3 years ago due to uncontrolled hypertension, left ventricular systolic dysfunction and approximately 70% stenosis of descending thoracic aorta. This lady was treated with embolization of hypertrophied bronchial artery as well as left internal mammary artery branch for management of haemoptysis. Embolization of hypertrophied bronchial artery in the setting of Takayasu arteritis with pulmonary arterial involvement presenting with haemoptysis is rare. Hypertrophied bronchial artery detected in post stenting computed tomography raises suspicion whether descending thoracic aorta stenting promotes the hypertrophy of bronchial artery. Literature of bronchial artery embolization in the setting of Takayasu with post DTA stenting is scarce.

Takayasu arteritis and hyperthyroidism: A secondary hypertension case report.

INTRODUCTION: Renovascular disease and hyperthyroidism are secondary hypertension. Takayasu arteritis (TAK) is a chronic, progressive, nonspecific great vasculitis involving the aorta and its major branches. It is one of the causes of renal artery stenosis. Hyperthyroidism is an endocrine disease caused by improper continuous synthesis and secretion of excessive thyroid hormone by the thyroid gland. Both diseases can raise blood pressure (BP). CASE PRESENTATION: we present a case of 18-year-old. Female, after exercise, fatigue palpitations. The maximum BP was 190/87 mm Hg, ankle-brachial index was <0.9. C-reactive protein and erythrocyte sedimentation rate were elevated. Imaging revealed multiple vascular stenosis. Triiodothyronine, tetraiodothyroxine, serum-free triiodothyronine, serum-free thyroxine, thyroid peroxidase antibody and thyroid stimulating receptor antibody were elevated. TSH reduced. She was diagnosed with TAK and hyperthyroidism. After treatment, the BP was normal, the thyroid function gradually returned to normal, and the symptoms improved. CONCLUSION: It is suggested that the BP of both upper limbs should be measured in newly diagnostic hypertension. If BP is not measured in both upper limbs, it is likely to be missed diagnosis. The cause of vascular stenosis needs to be identified, otherwise interventional treatment may lead to aggravation of the condition. Few cases of TAK complicated with hyperthyroidism have been reported. Both diseases are related to the immune system, whether there is any correlation between the 2 diseases, further research is needed. Early diagnosis, early treatment, the earlier intervention, the better prognosis.

[Takayasu's arteritis]. / Artérite de Takayasu.

TAKAYASU'S ARTERITIS. Takayasu's arteritis is an inflammatory panarteritis of the large vessels, preferentially affecting the aorta, its main branches, and the pulmonary arteries. Its incidence is estimated at 1.11 cases per million person-years, with a female predominance. The disease is classically characterized by the succession of two phases: a pre-occlusive inflammatory phase that may go unnoticed and an occlusive phase characterized by ischemic vascular symptoms because of parietal arterial lesions such as stenosis, occlusion or aneurysm. The diagnosis is based on clinical, biological and morphological findings. When available, pathological examination reveals a predominantly medial-adventitial, segmental and focal granulomatous panarteritis. Treatment consists of administering corticosteroid therapy and often immunosuppressants, or even biotherapies, managing cardiovascular risk factors, and managing vascular complications.

ARTÉRITE DE TAKAYASU. L'artérite de Takayasu est une panartérite inflammatoire des gros vaisseaux touchant préférentiellement l'aorte et ses branches principales ainsi que les artères pulmonaires. On estime son incidence annuelle à 1,11 cas par million de personnes, avec une prédominance féminine. Il est classiquement observé deux phases successives : une phase inflammatoire préocclusive pouvant passer inaperçue, puis une phase occlusive, caractérisée par des symptômes vasculaires ischémiques, conséquence des lésions artérielles pariétales à type de sténose, occlusion ou anévrisme. Le diagnostic repose sur un faisceau d'arguments cliniques, biologiques et morphologiques. Lorsqu'il est accessible, l'examen anatomopathologique retrouve un aspect de panartérite granulomateuse à prédominance médio-adventitielle, segmentaire et focale. Le traitement consiste en l'administration d'une corticothérapie et souvent d'immunosuppresseurs, ainsi qu'en la prise en charge des facteurs de risque cardiovasculaire afin de prévenir les complications vasculaires à plus long terme.

Angioplasty for Supra-Aortic Arterial Lesions from Takayasu Arteritis: Efficacy of Cutting Balloon Angioplasty Versus Conventional Balloon Angioplasty.

BACKGROUND: This retrospective study aimed to evaluate the safety and efficacy of cutting balloon angioplasty and conventional balloon angioplasty in supra-aortic arterial lesions caused by Takayasu arteritis. METHODS: A total of 46 patients with supra-aortic arterial lesions between January 2011 and December 2018 were included. Cutting balloon angioplasty was applied for 17 patients with 24 supra-aortic arterial lesions (group A), while 29 patients with 36 supra-aortic arterial lesions received conventional balloon angioplasty (group B). The preoperative clinical manifestation, operation result, and postoperative outcomes were recorded and compared in the 2 groups. RESULTS: Dizziness, visual disturbance, and unequal/absent pulses were the most common manifestations. The technical success of revascularization was 93.5% (43/46) in patients and 93.3% (56/60) in lesions. The stent implantation rate in group A was significantly lower than that in group B (4.2% vs. 50% in lesions, P < 0.05). Restenosis was the most common complication in both groups. Although the early (≤30 days) and late (>30 days) complications in group A were less than those in group B, there was no significant difference between the 2 groups (P > 0.05). Moreover, the primary-assisted patency of cutting balloon angioplasty and conventional balloon angioplasty at 1, 2, and 5 years were 66.7%, 62.5%, and 62.5% and 61.1%, 58.2%, and 49.8%, there was no significant difference between the 2 groups (P > 0.05), respectively. CONCLUSIONS: Compared with conventional balloon angioplasty, cutting balloon angioplasty could be considered a safe and effective alternative for supra-aortic arterial lesions caused by Takayasu arteritis, demonstrating better patency and clinical benefit.

The Treatment Dilemma of Arteriopathy in Takayasu Arteritis- A State-of-the-Art Approach.

Takayasu Arteritis (TA) is a chronic idiopathic granulomatous pan-arteritis affecting the pulmonary artery, the aorta, and its principal derived branches. The majority of TA patients are female (82.9%-97.0%). Due to the inflammatory character of the illness, arterial stenosis therapy must be treated differently than the atherosclerosis process. In this review paper, we outline a strategy using real-world challenging cases.

Cardiovascular Disease in Large Vessel Vasculitis: Risks, Controversies, and Management Strategies.

Takayasu's arteritis (TAK) and giant cell arteritis (GCA) are the 2 most common primary large vessel vasculitides (LVV). They share common vascular targets, clinical presentations, and histopathology, but target a strikingly different patient demographic. While GCA predominantly affects elderly people of northern European ancestry, TAK preferentially targets young women of Asian heritage. Cardiovascular diseases (CVD), including ischemic heart disease, cerebrovascular disease, aortic disease, and thromboses, are significantly increased in LVV. In this review, we will compare and contrast the issue of CVD in patients with TAK and GCA, with respect to prevalence, risk factors, and mechanisms of events to gain an understanding of the relative contributions of active vasculitis, vascular damage, and accelerated atherosclerosis. Controversies and possible mitigation strategies will be discussed.

Drug-Coated Balloon Angioplasty for Thoracic Great Vessel Stenosis Due to Takayasu Arteritis With 1-Year Follow-up.

PURPOSE: Takayasu arteritis is a large-vessel vasculitis in women of childbearing age that affects large vessels including the aorta and its main branches. Inflammation of arteries can produce lesions that lead to occlusion, stenosis, or aneurysms which can lead to complications. If signs of organ dysfunction are present, vascular intervention may be necessary. CASE REPORT: In this article, we present a case of Takayasu arteritis with high-grade stenosis of all the great vessels of the thoracic aorta treated with drug-coated balloon (DCB) angioplasty and stenting requiring multiple follow-up interventions over a 1-year follow-up. CONCLUSION: The DCB angioplasty is a potential endovascular treatment for thoracic great artery stenosis in Takayasu arteritis that could be further explored.

Epidemio-clinical profile of Takayasu Arteritis at the Center of Tunisia.

INTRODUCTION: Takayasu's Arteritis (TA) is a systemic vasculitis affecting the aorta and its main branches. AIM: To describe the epidemiological, diagnostic, therapeutic and prognostic profile of TA in the referral departments of internal medicine in the Sousse region (Tunisia). METHODS: This is a descriptive, retrospective and exhaustive study, carried out in the two departments of Internal Medicine of Sousse. Patients followed for AT, from 1996 to 2020 were included. The disease was defined according to the classification criteria of the American College of Rheumatology. Disease activity was assessed according to NIH criteria. Age referred to the date of diagnosis. RESULTS: The study population consisted of 40 patients (Sahloul: n=32, Hached: n=8) with a sex ratio=0.17 and a median age=35 years (IIQ=[30-41]). The median diagnostic delay was 5 months (IIQ=[2-14]). The main clinical sign was pulse abolition and/or decrease (78%). Aortic stenosis was the main arterial lesion found (98%). Treatment was based on corticosteroids (95%) and immunosuppressants (42%). The prognosis of TA was often active (62%), with vascular co-morbidity (60%) and iatrogenic complications (35%). CONCLUSION: The epidemiological-clinical profile of AT in the region of Sousse (Tunisia) was characterized by a female predominance, a diagnostic delay, a clinical polymorphism, and evolution towards vascular co-morbidities.

Inflammatory Diseases of the Aorta: JACC Focus Seminar, Part 2.

Inflammatory aortitis is most often caused by large vessel vasculitis (LVV), including giant cell arteritis, Takayasu's arteritis, immunoglobulin G4-related aortitis, and isolated aortitis. There are distinct differences in the clinical presentation, imaging findings, and natural history of LVV that are important for the cardiovascular provider to know. If possible, histopathologic specimens should be obtained to aide in accurate diagnosis and management of LVV. In most cases, corticosteroids are utilized in the acute phase, with the addition of steroid-sparing agents to achieve disease remission while sparing corticosteroid toxic effects. Endovascular and surgical procedures have been described with success but should be delayed until disease control is achieved whenever possible. Long-term management should include regular follow-up with rheumatology and surveillance imaging for sequelae of LVV.

Presentation and clinical course of pediatric-onset versus adult-onset Takayasu arteritis-a systematic review and meta-analysis.

Takayasu arteritis (TAK) is a less common large-vessel vasculitis which can occur in either children or adults. However, differences between pediatric-onset and adult-onset TAK have not been systematically analyzed. We undertook a systematic review (pre-registered on PROSPERO, identifier CRD42022300238) to analyze differences in clinical presentation, angiographic involvement, treatments, and outcomes between pediatric-onset and adult-onset TAK. We searched PubMed (MEDLINE and PubMed Central), Scopus, major recent international rheumatology conference abstracts, Cochrane database, and clinicaltrials.gov, and identified seven studies of moderate to high quality comparing pediatric-onset and adult-onset TAK. Meta-analysis of 263 pediatric-onset and 981 adult-onset TAK suggested that constitutional features (fever, and in subgroup analyses, weight loss), hypertension, headache, and sinister features of cardiomyopathy, elevated serum creatinine, and abdominal pain were more frequent in pediatric-onset TAK, whereas pulse loss/pulse deficit and claudication (particularly upper limb claudication) were more frequent in adult-onset TAK. Hata's type IV TAK was more common in pediatric-onset TAK, and Hata's type I TAK in adult-onset TAK. Children with TAK also appeared to require more intense immunosuppression with more frequent use of cyclophosphamide, biologic DMARDs, tumor necrosis factor alpha inhibitors, and, in subgroup analyses, tocilizumab in pediatric-onset TAK than in adult-onset TAK. Surgical or endovascular procedures, remission, and risk of mortality were similar in both children and adults with TAK. No studies had compared patient-reported outcome measures between pediatric-onset and adult-onset TAK. Distinct clinical features and angiographic extent prevail between pediatric-onset and adult-onset TAK. Clinical outcomes in these subgroups require further study in multicentric cohorts.